Background:Hypertrophic cardiomyopathy (HCM) is a genetic disease associated with risk of morbidity and sudden cardiac death. The prevalence, hypertrophy patterns, mode of presentations, and different ECG findings vary in different regions of the world. To date, no data is present regarding these variables in Qatar.Patients and Methods:A retrospective, cross sectional, descriptive analysis of all patients referred for echocardiography study at Hamad General Hospital, Qatar. The study period was from January 2008 till December 2010.Aims:To study 1) the prevalence of HCM, 2) the different patterns of hypertrophy, and 3) the clinical and ECG presentations in this population.Results:Out of the 29,286 cases evaluated, 38 patients were found to have HCM (0.13%). Their clinical, ECG, and echocardiography findings were analyzed. Mean age was 47 y, 35 males (92%) and 3 females (8%). Four patterns of hypertrophy were described; 17 (44.7%) had septal hypertrophy alone, 6 (15.8%) had septal and other segments hypertrophy but sparing the apex, 10 (26.3%) had apical segments along with any other segment hypertrophy, and 5 (13.2%) had apical hypertrophy alone. No obstruction was found in 19 (50%), left ventricular outflow (LVO) tract obstruction was found in 13 (34%), and mid cavity obstruction (MCO) in 6 (16%). Twenty one (55.3%) patients were referred because of chest pain, 15 (39.5%) with palpitations, 15 (39.5%) with shortness of breath, and 5 (13.2%) with syncope. Nine patients (23.7%) were asymptomatic and were referred because of cardiac murmur during routine examination. ECG evidence of LV hypertrophy was found in 29 (76.3%).Conclusion:The prevalence of HCM in our population group is 0.13% with a male predominance (12:1). There was a diversity of clinical presentation, ECG abnormalities and patterns of LV hypertrophy among HCM patients.

Conflicting data exist about the relationship between cardiac resynchronization therapy (CRT) and diastolic function.Aims of the study are to assess diastolic patterns in patients undergoing CRT according to the 2016 recommendations of the American Society of Echocardiography/European Association of Cardiovascular Imaging and to evaluate the prognostic value of diastolic dysfunction (DD) in CRT candidates. Methods and resultsOne-hundred ninety-three patients (age: 67±11 years, QRS width: 167±21 ms) were included in this multicentre prospective study. Mitral filling pattern, mitral tissue Doppler velocity, tricuspid regurgitation velocity, and indexed left atrial volume were used to classify DD from grade I to III.CRT-response, defined as a reduction of left ventricular (LV) end-systolic volume >15% at 6month follow-up (FU), occurred in 132 (68%) patients. The primary endpoint was a composite of heart transplantation, LV assisted device implantation, or all-cause death during FU and occurred in 29 (15%) patients.CRT was associated with a degradation of DD in non-responders. At multivariable analysis corrected for clinical variables, QRS duration, mitral regurgitation, CRT-response and LV dyssynchrony, grade I DD was associated with a better outcome (HR 0.37, 95% CI: 0.14-0.96).Non-responders with grade II-III DD had the worse prognosis (HR 4.36, 95%CI: 2.10-9.06). ConclusionsThe evaluation of DD in CRT candidates allows the prognostic stratification of patients, independently from CRT-response.

Background Ruptured sinus of Valsalva aneurysm (RSOVA) is rare, and it is more common in Asians. Typically, the patient presents with acute/subacute shortness of breath (SOB) and chest pain. Echocardiography is the gold standard for diagnosis in most of these cases. Surgery has remained the first line of management. Case summary We present two cases of RSOVA in which the patients presented to the emergency department with SOB. Their preoperative echocardiography results showed RSOVA into the right ventricle. During surgical repair, ventricular septal defect (VSD) was also found. Discussion RSOVA is frequently associated with other congenital anomalies, and most often with VSD. In our cases, we believe that VSDs were missed preoperatively because either the large aneurysmal sacs covered the VSD or there was overlap between the two shunts. Additionally, in the first case, right ventricular pressure was high approaching systemic pressure, which probably reduced the shunt across the VSD. Early intervention is recommended to prevent endocarditis or enlargement of the ruptured aneurysm; long-term results were excellent after surgical repair. Most patients undergo surgery between 20 and 40 years of age, and the reported survival rate is 95% at 20 years. If left untreated, patients typically die of heart failure or endocarditis within 1 year after onset of symptoms.

Funding Acknowledgements Type of funding sources: None. Background Despite having all a systolic heart failure and broad QRS, patients proposed for cardiac resynchronization therapy (CRT) are highly heterogeneous and it remains extremely complicated to predict the impact of the device on left ventricular (LV) function and outcomes. Objectives We sought to evaluate the relative impact of clinical, electrocardiographic, and echocardiographic data on the left ventricular (LV) remodeling and prognosis of CRT-candidates by the application of machine learning (ML) approaches. Methods 193 patients with systolic heart failure undergoing CRT according to current recommendations were prospectively included in this multicentre study. We used a combination of the Boruta algorithm and random forest methods to identify features predicting both CRT volumetric response and prognosis (Figure 1). The model performance was tested by the area under the receiver operating curve (AUC). We also applied the K-medoid method to identify clusters of phenotypically-similar patients. Results From 28 clinical, electrocardiographic, and echocardiographic-derived variables, 16 features were predictive of CRT-response; 11 features were predictive of prognosis. Among the predictors of CRT-response, 7 variables (44%) pertained to right ventricular (RV) size or function. Tricuspid annular plane systolic excursion was the main feature associated with prognosis. The selected features were associated with a very good prediction of both CRT response (AUC 0.81, 95% CI: 0.74-0.87) and outcomes (AUC 0.84, 95% CI: 0.75-0.93) (Figure 1, Supervised Machine Learning Panel). An unsupervised ML approach allowed the identifications of two phenogroups of patients who differed significantly in clinical and parameters, biventricular size and RV function. The two phenogroups had significant different prognosis (HR 4.70, 95% CI: 2.1-10.0, p < 0.0001; log –rank p < 0.0001; Figure 1, Unsupervised Machine Learning Panel). Conclusions Machine learning can reliably identify clinical and echocardiographic features associated with CRT-response and prognosis. The evaluation of both RV-size and function parameters has pivotal importance for the risk stratification of CRT-candidates and should be systematically assessed in patients undergoing CRT. Abstract Figure 1

BackgroundDespite all having systolic heart failure and broad QRS, patients screened for cardiac resynchronization therapy (CRT) are highly heterogeneous, and it remains extremely challenging to predict the impact of the device on left ventricular (LV) function and outcomes. ObjectivesWe sought to evaluate the relative impact of clinical, electrocardiographic, and echocardiographic data on the left ventricular (LV) remodeling and prognosis of CRT-candidates by the application of machine learning (ML) approaches. Methods193 patients with systolic heart failure undergoing CRT according to current recommendations were prospectively included in this multicentre study. We used a combination of the Boruta algorithm and random forest methods to identify features predicting both CRT volumetric response and prognosis.The model performance was tested by the area under the receiver operating curve (AUC). We also applied the K-medoid method to identify clusters of phenotypically similar patients. ResultsFrom 28 clinical, electrocardiographic, and echocardiographic-derived variables, 16 features were predictive of CRT response, and 11 features were predictive of prognosis.Among the predictors of CRT-response, 8 variables (50%) pertained to right ventricular (RV) size or function. Tricuspid annular plane systolic excursion was the main feature associated with prognosis.The selected features were associated with a particularly good prediction of both CRT response (AUC 0.81, 95% CI: 0.74-0.87) and outcomes (AUC 0.84, 95% CI: 0.75-0.93). An unsupervised ML approach allowed the identifications of two phenogroups of patients who differed significantly in clinical variables and parameters of biventricular size, and RV function. The two phenogroups had significant different prognosis (HR 4.70, 95%